Isolated vocal cord palsy secondary to a paraoesophageal hiatus hernia: a rare variant of Ortner’s syndrome

Background

Dysphonia is the most common presenting symptom of vocal cord palsy, characteristically with a breathy voice due to air escape, and sometimes choking episodes on swallowing due to reduced laryngeal sensation.1 Ortner’s syndrome (cardiovocal syndrome) is a rare cause of left recurrent laryngeal nerve paresis/palsy secondary to cardiovascular pathology/structural enlargement.2 It was first described by Ortner, an Austrian physician, who correlated left vocal cord palsy in 3 patients with left atrial enlargement secondary to mitral stenosis.2 Most cases of Ortner’s syndrome are due to left recurrent laryngeal nerve compression between the aorta and pulmonary artery in the aortopulmonary window.3 Variations of Ortner’s syndrome have also been described in patients with thoracic aortic aneurysm, enlarged pulmonary artery and aberrant subclavian artery.4 A hiatus hernia compressing the left recurrent laryngeal nerve at the aortopulmonary window is a variant of Ortner’s syndrome. Identification is vital as vocal cord palsy can impact on patients’ quality of life and increase the risk of aspiration pneumonia.

Case presentation

A woman in her 70s was referred to the outpatient otolaryngology clinic with a 5-week history of progressive dysphonia on a background of smoking 10–20 cigarettes per day. No odynophagia or dysphagia was reported. The patient denied any throat pain, with no reported choking episodes. She denied any neck lumps, previous laryngeal surgery or other chest-related symptoms. She had no weight loss or other systemic symptoms of note. She had a 5-year history of nocturnal reflux and gastritis like symptoms, which improved with omeprazole. Prior to presentation, she reported mild reflux-related symptoms. She had no history of head and neck cancer. A medical history included heart failure, previous transient ischaemic attacks (TIAs), osteoporosis, carotid artery stenosis, hypertension, chronic kidney disease and GORD.

Clinical examination of the oropharynx showed no abnormalities and neck examination demonstrated no lymphadenopathy, mass lesion or palpable thyroid nodules. A right-sided neck scar from previous carotid endarterectomy was noted. The patient was noted to have a weak, breathy voice and flexible laryngoscopy detected an immobile left vocal cord in the paramedian position, consistent with a left vocal cord paralysis. No mass lesion or other abnormality on examination of the larynx or neck were evident. There was no pooling of saliva, and the supraglottic region appeared normal. There was no evidence of any obvious subglottic abnormality.

Investigations

Subsequent urgent CT scanning of the neck, thorax and upper abdomen was undertaken.

This demonstrated asymmetry of vocal cord position, with the left cord medialised, and distension of the ipsilateral pyriform sinus, compatible with vocal cord palsy.

There were no culpable findings at the skull base and in particular, the jugular foramen was clear. No carotid sheath lesions were identified. There were no other abnormalities in the soft tissues of the neck.

CT scan of the thorax and upper abdomen showed a very large hiatus hernia containing stomach, transverse colon and extensive mesenteric fat (figure 1). This produced significant mass effect within the mediastinum, resulting in apposition of the pulmonary trunk and aortic arch, and obliteration of the usual fat plane in the aortopulmonary window, thought to account for compression of the traversing left recurrent laryngeal nerve. No focal pulmonary mass or thoracic lymphadenopathy was detected. There were no other significant thoracoabdominal abnormalities.

Differential diagnosis

In the clinical context of an elderly patient with a significant smoking history with new persistent dysphonia, a malignancy of the larynx or chest is high on the differential diagnosis list, and needs to be assessed primarily as the aetiology of the vocal cord palsy. Given the fact laryngeal endoscopy showed a left vocal cord palsy with no obvious exophytic lesions, a malignancy of the neck, lung or oesophagus must be considered.

Skull base pathology can also cause isolated vocal cord paresis or paralysis and this is important to be assessed radiologically.

An important differential to consider in the elderly population with evidence of either unilateral or bilateral vocal cord involvement with other neurological signs or symptoms is a central neurological condition such as degenerative disease, movement disorders or demyelinating disease.

Treatment

Given that the CT scan of the neck and chest highlighted no overt pathology in the neck or thorax, other than a hiatus hernia, and with a lack of symptoms to suggest oesophageal pathology that is, no dysphagia, odynophagia or weight loss, a presumed diagnosis of a compressive hiatus hernia was made.

The patient was subsequently reviewed in the ear, nose and throat (ENT) outpatient clinic to assess for the development of a clinically apparent cause for the vocal cord palsy, to ensure no further progression of the palsy and to ascertain that the patient was safe from an aspiration point of view.

As there were no symptoms or clinical/radiological signs of sinister disease, the decision was made that an examination under anaesthesia or further investigations were not warranted, and surveillance was appropriate.

Outcome and follow-up

The patient was reviewed in the outpatient ENT clinic 3 months after the initial consultation. Her dysphonia had resolved and objectively her voice sounded better with no breathy character detected. She felt well in herself and no new symptoms or complaints were reported. The result of the CT scan was explained to her and due to her resolution of symptoms, she was discharged back to the care of her general practitioner.